Alzheimer’s Disease Manifesting as Corticobasal Degeneration – Case Report

Česká verzia

Authors: S. Johanidesová ¹; R. Rusina ¹; P. Houška ²; J. Kellerihash2 ^{3,4 3,5}
Authors place of work: Neurologická klinika IPVZ a Thomayerova nemocnice, Praha ¹; Neurologické oddělení, Nemocnice Strakonice, a. s. ²; 3. LF UK v Praze ³; Radiodiagnostické oddělení, Nemocnice na Homolce, Praha ⁴; Oddělení patologie a molekulární medicíny, Thomayerova nemocnice, Praha ⁵
Published in the journal: Cesk Slov Neurol N 2012; 75/108(3): 373-377
Category: Case Report

Summary

Corticobasal degeneration is a rare neurodegenerative disease that belongs to the group of tauopathies is clinically manifested as a combination of extrapyramidal symptoms and cortical dysfunction affecting mainly the parietal areas. The definite diagnosis of corticobasal degeneration is based on neuropathological findings. For this reason, the term corticobasal syndrome tends to be used in surviving patients. The objective of our paper is to present a case of a patient with corticobasal syndrome as a peculiar manifestation of neuropathologically confirmed Alzheimer’s disease. Our patient showed significant cognitive impairment in visuospatial functions, apparent apraxia, frontal signs as well as early episodic memory impairment. Asymmetrical extrapyramidal features with left-sided prevalent akinesia and rigidity, apraxia on the left upper extremity and occasional myoclonus were also present. The pattern of neuropathological changes met the criteria of a fully developed neocortical stage of an early Alzheimer’s disease without any evidence of corticobasal degeneration. The main features evoking Alzheimer’s disease-associated corticobasal syndrome and enabling its differentiation from corticobasal degeneration were lower age at onset and myoclonus. In contrast to previously published data, our patient presented with an early episodic memory impairment suggesting a predominant involvement of the hippocampal and entorhinal cortices.

Key words:
corticobasal syndrome – corticobasal degeneration – Alzheimer’s disease – tauopathies

Zdroje

1. Bak TH, Caine D, Hearn VC, Hodges JR. Visuospatial functions in atypical parkinsonian syndromes. J Neurol Neurosurg Psychiatry 2006; 77(4): 454–456.

2. Litvan I, Cummings J, Mega M. Neuropsychiatric features of corticobasal Degeneration. J Neurol Neurosurg Psychiatry 1998; 65(5): 717–721.

3. Ling H, Sullivan SS, Holton JL, Revesz T, Massey LK, Williams DR et al. Does corticobasal degeneration exist? A clinicopathological re-evaluation. Brain 2010; 133(7): 2045–2057.

4. Josephs KA, Whitwell JL, Boeve BF, Knopman DS, Petersen RC, Hu WT et al. Anatomical differences between CBS-corticobasal degeneration and CBS-Alzheimer’s disease. Mov Disord 2010; 25(9): 1246–1252.

5. Alladi S, Xuereb J, Bak T, Nestor P, Knibb J, Patterson K, Hodges JR. Focal cortical presentations of Alzheimer’s disease. Brain 2007; 130 (10): 2636–2645.

6. Lezak MD, Howieson DB, Loring DW. Neuropsychological assessment. Oxford: Oxford University Press 2004.

7. Vranová H, Kaňovský P, Mareš J, Nevrlý M, Stejskal D, Nestrašil I et al. Laboratorní ukazatele neurodegenerace v likvoru a míra motorického postižení u Parkinsonovy nemoci: korelační studie. Cesk Slov Neurol N 2008; 71/104(3): 324–328.

8. Vališ M, Taláb R, Andrýs C, Štourač P, Masopust J, Kalnická D et al. Tau-protein, fosforylovaný tau-protein a beta-amyloidu42 v likvoru u demencí a roztroušené sklerózy. Cesk Slov Neurol N 2008; 71/104(3): 329–335.

9. Kim SK, Park KW, Kang DY, Cha JK, Kim SH, Kim JW. Severe episodic memory impairment in a patient with clinical features compatible with corticobasal degeneration. J Clin Neurol 2008; 4(2): 94–98.

10. Shelley BP, Hodges JR, Kipps CM, Xuereb JH, Bak TH.Is the pathology of corticobasal syndrome predictable in life? Mov Disord 2009; 24(11): 1593–1599.

11. Mathuranath PS, Xuereb JH, Bak T, Hodges JR. Corticobasal ganglionic degeneration and/or frontotemporal dementia? A report of two overlap cases and review of literature. J Neurol Neurosurg Psychiatry 2000; 68(3): 304–312.

12. Xie S, Libon DJ, Wang X, Massimo L, Moore P, Vesely L et al. Longitudinal Patterns of Semantic and Episodic Memory in Frontotemporal Lobar Degeneration and Alzheimer’s Disease. Int Neuropsychol Soc 2010; 16(2): 278–286.

13. Hillis EA. Naming and language production. In: Grossman M (ed). Behavioural/cognitive Neurology. AAN Continuum: Lifelong Learning in Neurology. Philadelphia: Lippincott Williams & Wilkins 2010; 16(4): 29–44.

14. Barkhof F, Fox NC, Bastos-Leite A, Scheltens P. Neuroimaging in Dementia. 1st ed. Berlin Heidelberg: Springer-Verlag 2011.

15. Wadia PM, Lang AE. The many faces of corticobasal degeneration. Parkinsonism Relat Disord 2007; 13 (Suppl 3): S336–S340.

16. Hu WT, Rippon GW, Boeve BF, Knopman DS, Petersen RC, Parisi JE et al. Alzheimer’s disease and corticobasal degeneration presenting as corticobasal syndrome. Mov Disord 2009; 24(9): 1375–1379.